Herbert W. Oechler

Inflammatory myofibrohistiocytic proliferation simulating sarcoma in children

The term “inflammatory myofibrohistiocytic proliferation” (IMP) has been proposed to replace the conventional designations of plasma cell granuloma and inflammatory pseudotumor. Three cases of extrapulmonary IMP in children are reported, including an intracerebral lesion which has been formerly undescribed. In children, IMP may be associated with microcytic hypochromic anemia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. In this clinical setting, differentiation of a rapidly growing but benign IMP from a bona fide sarcoma is of paramount importance.

Clostridial septicemia complicating the course of leukemia

The authors report an analysis of 47 leukemia patients (including 9 from our own medical center) whose courses were complicated by 48 episodes of clostridial septicemia. There were 36 adults and 11 children; acute myelogenous leukemia and acute lymphoblastic leukemia accounted for 61.7% and 14.9% of cases, respectively. All patients for whom remission status was known were in leukemic relapse. Fever was a presenting complaint in at least 36 patients whereas neutropenia, thrombocytopenia, and gastrointestinal lesions were noted in 100%, 90.9%, and 87.9%, respectively, of the patients for whom information on these parameters was available. Overall mortality from clostridial septicemia was 78%; none of the children and none of the patients with intravascular hemolysis survived. Overall, antibiotic therapy resulted in a 40% survival rate. However, among patients receiving beta lactam and/or chloramphenicol therapy, 57% survived their episode of clostridial septicemia. Prompt initiation of appropriate antimicrobial therapy offers the best chance of survival in leukemia patients with clostridial septicemia.

Crohn's disease presenting as unilateral labial hypertrophy

We describe a child who had unilateral hypertrophy of the vulva, a rare extraintestinal manifestation of Crohns disease. A biopsy specimen revealed a noncaseating granuloma. Flexible sigmoidoscopic examination revealed punctate 1 mm lesions with surrounding erythema in the rectum and sigmoid colon. Response to treatment with sulfasalazine was excellent. Gastrointestinal symptoms developed 1 year later when sulfasalazine was discontinued.

Kimura’s disease: an unusual cause of cervical tumour

An 11 year old Chinese boy developed a unilateral cervical mass associated with pronounced eosinophilia and a marked increase in IgE concentrations. A biopsy sample showed massive eosinophilic tissue infiltration consistent with Kimura’s disease. This disorder should be suspected when the clinical triad of painless unilateral cervical adenopathy, hypereosinophilia, and hyper-IgE is present, particularly in male Asian patients.

Hepatic ultrastructure in leukemic children treated with methotrexate and 6-mercaptopurine.

Methotrexatc (MTX) and 6-mercaptopurine (6MP), the two drugs most commonly used for maintenance treatment of childhood leukemia, are hoth potent hepatotoxins. In order to assess MTX-6MP-induced damage, we obtained biopsies from 11 children with acute lymphocytic leukemia (ALL) for light microscopic and transmission electron microscopic study. Prednisone, vincristine, and L-asparaginase were used for induction of remission in all patients. Although light microscopic findings were minimal, we found significant ultrastructural abnormalities in all patients. Changes included nuclear abnormalities, disruption of rough and smooth endoplasmic reticulum, a variety of mitochondrial changes, steatosis, fibrosis, and changes in peroxisomes and lysosomes. These abnormalities could not have been predicted from liver function tests or histopathology. Three of the eleven patients studied had also received cyclophosphamide and cytosine arabinoside during maintenance therapy. The ultrastructural abnormalities in this group were not distinguishable from those observed in the group that did not receive these additional chemotherapeutic agents. The long-term clinical significance of these findings is not known.

Thyroid carcinoma following treatment for acute lymphoblastic leukemia.

A 21/2‐year‐old girl with acute lymphoblastic leukemia received chemotherapy and prophylactic cranial irradiation. After six years of remission, including three years off therapy, metastatic thyroid carcinoma appeared in the cervical lymph nodes. The predisposing factors for the development of thyroid carcinoma as a second malignancy in this case are discussed. It is suggested that thyroid carcinoma should be added to the growing list of second malignancies in acute lymphoblastic leukemia and that careful thyroid examination be included in the follow‐up of long‐term survivors.

Anesthesia-induced rhabdomyolysis in infants with unsuspected Duchenne dystrophy

Anesthesia‐induced rhabdomyolysis in infancy may represent an unsuspected Duchenne dystrophy. In order to establish the diagnosis of this genetic disease more definitively, a dystrophin test is a requisite following the conventional creatine kinase test and light and electron microscopies of the muscle biopsy.

Isolated bilateral T-cell renal lymphoblastic lymphoma.

A 3-year-old girl presented with bilateral renal masses. The histologic diagnosis was lymphoblastic lymphoma. Cell surface markers showed a cytotoxic/suppressor T cell phenotype. The patient was treated with APO therapy. She has remained continuously disease-free for 30 months and has been off all therapy for 6 months. This rare case supports the value of precise classification (by histologic and immunologic methods) as a guide to appropriate tumor therapy.

Effectiveness of a six-drug regimen (APO) without local irradiation for treatment of mediastinal lymphoblastic lymphoma in children.

Fourteen children with mediastinal lymphoblastic lymphoma (MLL) were treated with the six‐drug APO protocol. This regimen includes aggressive intermittent chemotherapy and prophylactic central nervous system therapy. Mediastinal irradiation was given only for emergency relief of mediastinal compression (two children) or for incomplete resolution of mediastinal widening with chemotherapy (one child). All 14 patients achieved complete remission; only 1 has relapsed. Toxicities were not severe. APO is effective therapy for children with MLL. APO should receive further trials in children and adults with T‐cell lymphoblastic lymphomas.

Circulatory Physiology in Isolated Pulmonary Hypoplasia: Persistence of Fetal Circulation

A case of congenital isolated pulmonary hypoplasia is presented. The circulatory physiology is reviewed. With the very high pulmonary resistance, there is obligatory right-to-left shunting through a ductus arteriosus, and the circulation thus resembles that of the fetal state.